Von During reports a curious case which he calls sclerodactylia annularis ainhumoides. The patient was a boy about twelve years old, born in Erzeroum, brought for treatment for scabies, and not for the affection about to be described. A very defective history led to the belief that a similar affection had not been observed in the family. When he was six years old it began on the terminal phalanges of the middle fingers. A myxomatous swelling attacked the phalanges and effected a complete absorption of the terminal phalanx. It did not advance as far as gangrene or exfoliation of bone. At the time of report the whole ten fingers were involved; the bones seemed to be thickened, the soft parts being indurated or sclerosed. In the right index finger a completely sclerosed ring passed around the middle phalanx. The nails on the absorbed phalanges had become small and considerably thickened plates. No analogous changes were found elsewhere, and sensation was perfectly normal in the affected parts. There were no signs whatever of a multiple neuritis nor of a leprous condition.
There is a rare and curious condition known as "deciduous skin" or keratolysis, in which the owners possess a skin, which, like that of a serpent, is periodically cast off, that of the limbs coming off like the finger of a glove. Preston of Canterbury, New Zealand, mentions the case of a woman who had thus shed her skin every few weeks from the age of seven or even earlier. The woman was sixty-seven years of age; the skin in every part of the body came away in casts and cuticles which separated entire and sometimes in one unbroken piece like a glove or stocking. Before each paroxysm she had an associate symptom of malaise. Even the skin of the nose and ears came off complete. None of the patient's large family showed this idiosyncrasy, and she said that she had been told by a medical man that it had been due to catching cold after an attack of small-pox. Frank mentions a case in which there was periodic and complete shedding of the cuticle and nails of the hands and feet, which was repeated for thirty-three consecutive years on July 24th of each year, and between the hours of 3 P.M. and 9 P.M. The patient remembered shedding for the first time while a child at play. The paroxysms always commenced abruptly, constitutional febrile symptoms were first experienced, and the skin became dry and hot. The acute symptoms subsided in three or four hours and were entirely gone in twelve hours, with the exception of the redness of the skin, which did not disappear for thirty-six hours more. The patient had been delirious during this period. The cuticle began to shed some time between the third and twelfth day, in large sheets, as pictured in the accompanying illustrations. The nails were shed in about four weeks after the acute stage. Crocker had an instance of this nature in a man with tylosis palmae, in which the skin was cast off every autumn, but the process lasted two months. Lang observed a case in which the fingers alone were affected.
There is a case of general and habitual desquamation of the skin in the Ephemerides of 1686; and Newell records a case which recovered under the use of Cheltenham water for several seasons. Latham describes a man of fifty who was first seized about ten years previously with a singular kind of fever, and this returned many times afterward, even twice in the course of the same year, attended with the same symptoms and circumstances, and appearing to be brought on by obstructed perspiration, in consequence of catching cold. Besides the common febrile symptoms, upon the invasion of the disease his skin universally itched, more especially at the joints, and the itching was followed by many little red spots, with a small degree of swelling. Soon after this his fingers became stiff; hard, and painful at the ends, and at the roots of the nails. In about twenty-four hours the cuticle began to separate from the cutis, and in ten or twelve days this separation was general from head to foot, during which time he completely turned the cuticle off from the wrists to the fingers' ends like a glove, and in like manner on the legs to the toes, after which his nails shot gradually from their roots, at first with exquisite pain, which abated as the separation of the cuticle advanced, and the old nails were generally thrown off by new ones in about six months. The cuticle rose in the palms and soles like blisters, having, however, no fluid beneath, and when it came off it left the underlying cutis exposed for a few days. Sometimes, upon catching cold, before quite free from feverish symptoms, a second separation of the cuticle from the cutis occurred, but it appeared so thin as to be like scurf, demonstrating the quick renewal of the parts.
There is a similar case in the Philosophical Transactions in a miller of thirty-five who was exposed to great heat and clouds of dust. On the first cold a fever attacked him, and once or twice a year, chiefly in the autumn, this again occurred, attended with a loosening and detachment of the cuticle. The disorder began with violent fever, attended with pains in the head, back, limbs, retching, vomiting, dry skin, furred tongue, urgent thirst, constipation, and high-colored urine. Usually the whole surface of the body then became yellow. It afterward became florid like a rash, and then great uneasiness was felt for several days, with general numbness and tingling; the urine then began to deposit a thick sediment. About the third week from the first attack the cuticle appeared elevated in many places, and in eight or ten days afterward became so loose as to admit of its easy removal in large flakes. The cuticle of the hands, from the wrists to the fingers' ends, came off like a glove. The patient was never disposed to sweat, and when it was attempted to force perspiration he grew worse; nor was he much at ease until his urine deposited a sediment, after which he felt little inconvenience but from the rigidity of the skin. The nails were not detached as in the previous case.
It is quite natural that such cases as this should attract the attention of the laity, and often find report in newspapers. The following is a lay-report of a "snake-boy" in Shepardstown, Va.:—
"Jim Twyman, a colored boy living with his foster-parents ten miles from this place, is a wonder. He is popularly known as the "snake-boy." Mentally he is as bright as any child of his age, and he is popular with his playmates, but his physical peculiarities are probably unparalleled. His entire skin, except the face and hands, is covered with the scales and markings of a snake. These exceptions are kept so by the constant use of Castile soap, but on the balance of his body the scales grow abundantly. The child sheds his skin every year. It causes him no pain or illness. From the limbs it can be pulled in perfect shape, but off the body it comes in pieces. His feet and hands are always cold and clammy. He is an inordinate eater, sometimes spending an hour at a meal, eating voraciously all the time, if permitted to do so. After these gorgings he sometimes sleeps two days. There is a strange suggestion of a snake in his face, and he can manipulate his tongue, accompanied by hideous hisses, as viciously as a serpent."
Under the name of dermatitis exfoliativa neonatorum, Ritter has described an eruption which he observed in the foundling asylum at Prague, where nearly 300 cases occurred in ten years. According to Crocker it begins in the second or third week of life, and occasionally as late as the fifth week, with diffuse and universal scaling, which may be branny or in laminae like pityriasis rubra, and either dry or with suffusion beneath the epidermis. Sometimes it presents flaccid bullae like pemphigus foliaceus, and then there are crusts as well as scales, with rhagades on the mouth, anus, etc.; there is a total absence of fever or other general symptoms. About 50 per cent die of marasmus and loss of heat, with or without diarrhea. In those who recover the surface gradually becomes pale and the desquamation ceases. Opinions differ regarding it, some considering it of septic origin, while others believe it to be nothing but pemphigus foliaceus. Kaposi regards it as an aggravation of the physiologic exfoliation of the new-born. Elliott of New York reports two cases with a review of the subject, but none have been reported in England. Cases on the Continent have been described by Billard, von Baer, Caspary, those already mentioned, and others.
The name epidemic exfoliative dermatitis has been given to an epidemic skin-disease which made its appearance in 1891 in England; 425 cases were collected in six institutions, besides sporadic cases in private houses.
In 1895, in London, some photographs and sketches were exhibited that were taken from several of the 163 cases which occurred in the Paddington Infirmary and Workhouse, under the care of Dr. Savill, from whose negatives they were prepared. They were arranged in order to illustrate the successive stages of the disorder. The eruption starts usually with discrete papules, often in stellate groups, and generally arranged symmetrically when on the limbs. These become fused into crimson, slightly raised maculae, which in severe cases become further fused into red thickened patches, in which the papules can still be felt and sometimes seen. Vesicles form, and exudation occurs in only about one-third of the cases. Desquamation of the epidermis is the invariable feature of all cases, and it usually commences between the fourth and eighth days. In severe cases successive layers of the epidermis are shed, in larger or smaller scales, throughout the whole course of the malady. One-half of the epidermis shed from the hand of a patient is exhibited in this collection.
Of sphaceloderma, or gangrene of the skin, probably the most interesting is Raynaud's disease of symmetric gangrene, a vascular disorder, which is seen in three grades of intensity: there is local syncope, producing the condition known as dead-fingers or dead-toes, and analogous to that produced by intense cold; and local asphyxia, which usually follows local syncope, or may develop independently. Chilblains are the mildest manifestation of this condition. The fingers, toes, and ears, are the parts usually affected. In the most extreme degree the parts are swollen, stiff, and livid, and the capillary circulation is almost stagnant; this is local or symmetric gangrene, the mildest form of which follows asphyxia. Small areas of necrosis appear on the pads of the fingers and of the toes; also at the edges of the ears and tip of the nose. Occasional symmetric patches appear on the limbs and trunk, and in extensive cases terminate in gangrene. Raynaud suggested that the local syncope was produced by contraction of the vessels; the asphyxia is probably caused by a dilatation of the capillaries and venules, with persistence of the spasm of the arterioles. According to Osler two forms of congestion occur, which may be seen in adjacent fingers, one of which may be swollen, intensely red, and extremely hot; the other swollen, cyanotic, and intensely cold. Sometimes all four extremities are involved, as in Southey's case, in a girl of two and a half in whom the process began on the calves, after a slight feverish attack, and then numerous patches rapidly becoming gangrenous appeared on the backs of the legs, thighs, buttocks, and upper arms, worse where there was pressure; the child died thirty-two hours after the onset. The whole phenomenon may be unilateral, as in Smith's case, quoted by Crocker,—in a girl of three years in whom the left hand was cold and livid, while on the right there was lividity, progressing to gangrene of the fingers and of the thumb up to the first knuckles, where complete separation occurred.